LAUSR

The embryology of the external male genitalia has been variably defined. This is partly due to extrapolation of observations made in animal models to human embryology. The genital tubercle appears in human embryos by the sixth week. Based on mouse models, it has been suggested that during genital tubercle formation the infra-umbilical mesoderm extends ventrally. With this the membranous cloaca undergoes marked reorientation and extends distally into the genital tubercle as the phallic cloaca. Smooth muscle and epithelial differentiation in the human foetal penis may be dependent on reciprocal cell–cell interactions, as is observed in other organs (e.g. bladder and prostate). Primary continuous urinary incontinence is a rare problem in association with hypospadias. We report two cases of complex hypospadias with penile ectopia where continuous urinary incontinence is the primary problem. Case 1: A 9-year-old boy presented with a history of proximal hypospadias. His initial management was at another centre and his original abnormality was a perineal hypospadias with chordee, a small phallus and complete penoscrotal transposition. His karyotype was confirmed as 46XY without mosaicism. He required multiple further procedures including urethral calibrations and closure of urethrocutaneous (perineal) fistula. A two-stage redohypospadias repair was subsequently performed. He was referred to our unit with primary continuous incontinence resulting in excoriation of the scrotum. He was still able to void on command. His work-up included a detailed bladder functional assessment. Micturating cystourethrogram revealed an open bladder neck. Cystoscopy confirmed an open bladder neck and showed bilateral medially ectopic ureters with a poorly developed trigone. Subsequently, he underwent a modified Kelly procedure, bladder neck reconstruction, bilateral ureteric reimplantation and correction of penoscrotal transposition. Figure 1a shows the anatomy of the external genitalia with residual incomplete penoscrotal transposition, and Figure 1b shows the open bladder with ectopic ureters and poorly developed trigone. The bladder neck reconstruction was made particularly challenging by: the bladder neck being a deep pelvic organ in the older child, the proximity of the repair to ectopic ureters, and a posteriorly placed proximal urethra. Because of poor outflow resistance related to the bladder neck malformation he had not developed normal bladder capacity. The option of an initial bladder neck reconstruction was suggested, but the patient preferred to proceed directly to bladder augmentation with bladder neck reconstruction. Case 2: An 11-year-old boy with severe penoscrotal hypospadias was referred to our unit with primary continuous incontinence. He had continuous urine leakage but was still able to pass some urine on command. He had multiple previous procedures but the details of these were not available. The penis remained highly ectopic; arising from the ischiopubic rami with very short corpora. Video urodynamics confirmed an extremely small capacity bladder and a widely open bladder neck during the filling phase (Fig. 2). Cystoscopy showed a malformed trigone extending into the prostatic region. The right ureteric orifice was particularly medially ectopic, opening close to the verumontanum. He subsequently underwent bladder neck reconstruction, bladder augmentation and mitrofanoff formation with right ureteric reimplantation. Subsequently, he underwent successful injection of Macroplastique (Congentic Medical, Geleen, The Netherlands) to the bladder neck due to ongoing minor leakage. Perineal phallus, ectopic penis, posterior ectopia of penis, prepenile scrotum or complete penoscrotal transposition are all terms that describe a rare and unusual urogenital malformation. The embryological sequence responsible for these malformations is